Clinical Immunology Flashcards

0
Q

SPUR

A
Suspect immunocompromised if any of...
Severe infection
Persistent infection
Unusual infecting agent
Recurrent infection
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1
Q

Hyper IgM

A

Group of immunodeficiency syndromes characterised by failure to class switch
Therefore high or normal levels IgM - low levels IgG / IgA
Antenatal diagnosis possible
Relatives of T cell immunodeficiency

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2
Q

Classification of immunodeficiencies

A

Primary - defects on gene(s) - even if unknown

Secondary - acquired - environmental factors

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3
Q

Most immunodeficiencies…

A

NOT primary
Approx 1/100,000 have primary immunodeficiency
Most secondary, often doctors are direct cause

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4
Q

Secondary immunodeficiency causes

A
Infections eg HIV
Malignancies (and their treatments)
Extremes of age
Drugs - toxins, corticosteroids
Chronic disease and nephrotic syndrome
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5
Q

Drugs as immunosuppressants

A

Cyclosporine, tacrolimus, rapamycin
Anti - TNFa therapies
Depleting mAb - rituximab
Chronic lymphocytic leukaemia treatment induces AIDS like state

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6
Q

Recurrent infections which may NOT imply identifiable immunodeficiency

A
Otitis media (>5 years as cut off)
UTI
Group A streptococcus
Staphylococcal furuncles
Dental carries
HSV (genital)
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7
Q

Indications for investigation immunocompromised

A
>2 systemic bacterial infections
>3 respiratory / other specific site bacterial infection
Infection at unusual site
Infection with unusual pathogen
Unusually severe infection with low virulence organisms / unusual complication
Past surgery for infection
>2 months antibiotics with little effect
Failure to thrive in infancy
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8
Q

Classification of primary immunodeficiency

A
Almost half are just antibody
About 1/4 phagocytes
Some cellular
Some complement
About 1/4 combined
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9
Q

B cell deficiency can result in

A

Bacterial infections of all types

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10
Q

T cell deficiency results in…

A

Mainly viruses, fungi, intracellular bacteria

Bacterial infections if severe

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11
Q

Complement deficiency results in…

A

Encapsulated bacteria
Eg neisseria meningitides
Haemophilia influenzae
Streptococcus pneumoniae

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12
Q

Phagocyte deficiency results in…

A

Fungi
Mycobacteria
Specific bacteria inc. salmonella, burkholderia

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13
Q

IgA deficiency

A

1:700 (Caucasians)
Respiratory, gut infection, coeliac disease
Major reactions to blood product
Worse if also IgG subclass deficiency

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14
Q

IgG subclass deficiency

A

IgG1/3 - effects response to protein antigens
IgG2 - response to polysaccharides
IgG4 - no consensus on significance, normal range can be 0

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15
Q

Common variable immunodeficiency (CVID) prevelence

A

Heterogenous group disorders
Core feature IgG deficiency
20-50 per million
Lifelong, 20% have family history

16
Q

CVID disease manifestations

A

Recurrent sino-pulmonary infections
Granulomatous disease
Autoimmunity & cytopenias

17
Q

X linked agammaglobulinaemia

A

Defect in Bruton’s tyrosine kinase, no heavy chain rearrangement
No B cells,lymphoid tissue, immunoglobulin
Present at 6 months +, antenatal diagnosis possible
Total - absence of Igs and antibodies

18
Q

Infections in antibody deficient states - recurrent and persistent

A

Recurrent - otitis media, sinusitis, pneumonia, bacteraemia

Persistent - rotavirus, girsrdia, enterovirus

19
Q

Transient infantile hypogammaglobulinaemia

A

Dip around 4 months, corrected by 6 months
Decrease in maternal IgG, while still low rate of endogenous production
Normal

20
Q

Treating antibody deficiency

A

Immunoglobulin transfusion - pooled, screened

Iv or subcutaneous

21
Q

Severe combined immunodeficiency (SCID)

A

Unwell by 3 months
Persistent diarrhoea, failure to thrive
Infections of all types inc. unusual skin disease
Family history of early infant death

22
Q

DiGeorge Syndrome

A

Pure T cell defect
Branchial arch defects
- hypoparathyroidism, aortic arch defects, thymol aplasia, dysmorphic
Translocation chromosome 22

23
Q

PNP deficiency

A

Pure T cell deficiency

Progressive decrease T cell numbers

24
Q

Pure T cell defects and SCID…

A

Infections - candida, mycobacterium, cryptosporidium
Failure to thrive, rashes
Family history / consanguinity
NO LIVE VACCINES

25
Q

Treating SCID

A

Bubble
Bone marrow transplant
Gene therapy (some leukaemia)

26
Q

Deficiencies in terminal complement components

A

(c5-8)

Characterised by recurrent Neisserial infections

27
Q

Cytokines defects

A

Present as typical/atypical mycobacterial infections
Requires in vitro cytokine assays for diagnosis
Some respond to gamma interferon

28
Q

Type one sensitivity

A

Immediate

IgE / mast cell mediated

29
Q

Type II hypersensitivity

A

Antibody mediated - IgM, IgG, opsonisation, phagocytosis

30
Q

Type III hypersensitivity

A

Immune complex mediated

Antigens, IgM, IgG, complement, activation of leukocytes

31
Q

Type IV hypersensitivity

A

CD4+ T cells - cytokine mediated inflammation

CD8+ CTLs - T cell mediated lysis

32
Q

Management of type one hypersensitivites

A

Atopic asthma, excema, atopic rhinitis, food allergies
Anaphylactic shock
Skin testing, drug therapies - antihistamines, epinephrine, desensitisation